Rheumatology and Immunology Practice Questions

Q: All may be associated with Sicca syndrome except?

Midline granuloma. **Explanation:** **Sicca Syndrome** (Primary Sjögren’s Syndrome) is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, primarily leading to xerophthalmia (dry eyes) and xerostomia (dry mouth). It can occur alone or secondary to other connective tissue diseases. **Why Midline Granuloma is the correct answer:** Midline granuloma (often referring to **Lethal Midline Granuloma** or Granulomatosis with Polyangiitis) is a destructive inflammatory process involving the nasal passage and paranasal sinuses. It is characterized by necrotizing granulomas and vasculitis. It does not typically involve the lymphocytic infiltration of salivary or lacrimal glands and is therefore not associated with Sicca syndrome. **Analysis of other options:** * **Scleroderma (Systemic Sclerosis):** Frequently associated with secondary Sjögren’s syndrome due to glandular fibrosis and autoimmune destruction. * **Rheumatoid Arthritis (RA):** This is the **most common** autoimmune disease associated with secondary Sjögren’s syndrome (occurring in ~30% of RA patients) [1]. * **Lymphadenopathy:** Patients with Sjögren’s syndrome often exhibit lymph node enlargement. Crucially, they have a **40-fold increased risk** of developing B-cell Non-Hodgkin Lymphoma (MALToma), making lymphadenopathy a significant clinical finding to monitor. **High-Yield Clinical Pearls for NEET-PG:** * **Antibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are the hallmark markers. * **Diagnosis:** Schirmer’s test (measures tear production) and Lip biopsy (showing focal lymphocytic sialadenitis) are gold standards. * **Extraglandular manifestation:** Raynaud’s phenomenon and cutaneous vasculitis are common. * **Highest Risk:** Sjögren’s syndrome carries the highest risk of lymphoma among all autoimmune diseases.

Q: Which of the following is NOT a feature of seronegative spondyloarthropathy?

Symmetrical Polyarthritis. **Explanation:** Seronegative spondyloarthropathies (SpA) are a group of inflammatory diseases (including Ankylosing Spondylitis, Psoriatic Arthritis, Reactive Arthritis, and Enteropathic Arthritis) characterized by the absence of Rheumatoid Factor (RF) and distinct clinical features [1]. **Why "Symmetrical Polyarthritis" is the correct answer:** Seronegative spondyloarthropathies typically present as **Asymmetrical Oligoarthritis** (involving <5 joints, often in the lower limbs) [1]. In contrast, **Symmetrical Polyarthritis** is the hallmark of Rheumatoid Arthritis [2]. While Psoriatic Arthritis can occasionally present with a symmetrical pattern, it is not a defining or universal feature of the SpA group. **Analysis of Incorrect Options:** * **A. Strong association with HLA B27:** This is a classic feature. HLA-B27 is most strongly associated with Ankylosing Spondylitis (>90% of cases) and is a key diagnostic marker for the entire group [1]. * **B. Negative Rheumatoid Factor:** By definition, these conditions are "seronegative," meaning RF and Anti-CCP antibodies are typically absent [1]. * **D. Enthesitis:** This is a pathognomonic feature of SpA. It refers to inflammation at the site where tendons or ligaments insert into bone (e.g., Achilles tendonitis or plantar fasciitis) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Common Features (PEAR):** **P**soriatic arthritis, **E**nteropathic arthritis, **A**nkylosing spondylitis, **R**eactive arthritis. * **Axial Involvement:** Sacroiliitis and spondylitis (bamboo spine) are characteristic [1]. * **Extra-articular manifestations:** Acute anterior uveitis is the most common extra-articular finding [1]. * **Dactylitis:** "Sausage digits" are frequently seen, especially in Psoriatic and Reactive arthritis.

Q: Which of the following statements about fibromyalgia is true?

SPECT studies have shown reduced blood flow to the thalamus. Explanation: Fibromyalgia is a chronic pain syndrome characterized by widespread musculoskeletal pain, fatigue, and sleep disturbances [1]. It is considered a disorder of central pain processing (central sensitization). Why Option D is Correct: Functional neuroimaging studies, including SPECT (Single-Photon Emission Computed Toxicity) and functional MRI, have consistently demonstrated altered pain processing in the brain [1]. Specifically, patients with fibromyalgia show reduced regional cerebral blood flow (hypoperfusion) in the thalamus and the caudate nucleus [1]. Since the thalamus acts as the "gatekeeper" for sensory input, this dysfunction contributes to the lowered pain threshold (allodynia). Analysis of Incorrect Options: * Option A: Fibromyalgia is significantly more common in females (ratio approx. 7:1 to 9:1), typically presenting between ages 20 and 50. * Option B: Sleep studies (Polysomnography/EEG) show a characteristic disruption in Stage 4 Non-REM sleep, specifically the "alpha-delta sleep" pattern (intrusion of alpha waves into deep delta sleep), rather than REM sleep disruption [1]. * Option C: Fibromyalgia is associated with a blunted (decreased) cortisol response to stress due to mild secondary adrenal insufficiency or hypothalamic-pituitary-adrenal (HPA) axis dysregulation [1], not an increased response. High-Yield Clinical Pearls for NEET-PG: * Diagnosis: Primarily clinical [1]. The 2010/2016 ACR criteria focus on the Widespread Pain Index (WPI) and Symptom Severity (SS) scale, moving away from the older "11 of 18 tender points" requirement. * Lab Findings: All inflammatory markers (ESR, CRP) and muscle enzymes (CPK) are characteristically normal [2]. * Management: First-line treatment involves patient education and aerobic exercise [2]. Pharmacotherapy includes Amitriptyline (TCA), Duloxetine (SNRI), or Pregabalin (Gabapentinoid).

Q: A 24-year-old male presents with abdominal pain, rashes, palpable purpura, and arthritis. What is the most probable diagnosis?

Henoch-Schönlein Purpura (HSP). ### Explanation **Correct Answer: A. Henoch-Schönlein Purpura (HSP)** **Clinical Reasoning:** Henoch-Schönlein Purpura (now commonly termed **IgA Vasculitis**) is a small-vessel leukocytoclastic vasculitis characterized by the deposition of **IgA-dominant immune complexes**. The classic clinical tetrad presented in this case is pathognomonic: 1. **Palpable Purpura:** Non-thrombocytopenic, typically found on gravity-dependent areas (lower limbs/buttocks). 2. **Arthritis/Arthralgia:** Usually migratory and affecting large joints (knees/ankles). 3. **Abdominal Pain:** Due to bowel wall edema or intussusception. 4. **Renal Involvement:** Ranging from hematuria to glomerulonephritis. **Why other options are incorrect:** * **Sweet Syndrome:** An acute febrile neutrophilic dermatosis characterized by painful, erythematous plaques/nodules and fever, usually associated with malignancy or infections, rather than the IgA-mediated systemic tetrad. * **Meningococcemia:** Presents with a rapidly progressing petechial/purpuric rash and high-grade fever. Patients appear acutely toxic/septic with signs of meningitis or DIC, which is absent here. * **Kawasaki Disease:** Primarily affects children <5 years. Key features include prolonged fever, conjunctivitis, strawberry tongue, and coronary artery aneurysms, not the purpura-arthritis-abdominal pain triad. **High-Yield Pearls for NEET-PG:** * **Most common** systemic vasculitis in childhood (though it can occur in adults). * **Trigger:** Often follows an Upper Respiratory Tract Infection (URTI). * **Biopsy:** Shows leukocytoclastic vasculitis with **IgA and C3 deposition** on immunofluorescence. * **Complication:** Intussusception in HSP is typically **ileo-ileal** (unlike the common idiopathic ileo-colic type). * **Prognosis:** Generally excellent; long-term morbidity is determined by the severity of **renal involvement**.

Q: Anti-fibrillarin antibodies are associated with which condition?

Systemic Sclerosis. **Anti-fibrillarin antibodies** (also known as **anti-U3 RNP**) are highly specific markers for **Systemic Sclerosis (SSc)**, particularly the **diffuse cutaneous subtype**. Fibrillarin is a major component of the nucleolus involved in pre-rRNA processing. On indirect immunofluorescence, these antibodies typically produce a **clumpy nucleolar pattern**. Clinically, anti-fibrillarin antibodies are associated with younger age of onset, frequent internal organ involvement (especially pulmonary hypertension and skeletal muscle involvement), and a generally poorer prognosis [1]. **Analysis of Incorrect Options:** * **A. Rheumatoid Arthritis:** Characterized by **Anti-CCP** (most specific) and Rheumatoid Factor (RF). It does not involve nucleolar antigens like fibrillarin [1]. * **B. Systemic Lupus Erythematosus (SLE):** Associated with **Anti-dsDNA** (specific/prognostic) and **Anti-Smith** (most specific). While ANA is positive, the patterns are usually homogeneous or speckled, not clumpy nucleolar [1]. * **C. Mixed Connective Tissue Disease (MCTD):** Defined by high titers of **Anti-U1 RNP** antibodies [1]. While it shares features of SSc, anti-fibrillarin is not a diagnostic marker for MCTD. **High-Yield Clinical Pearls for NEET-PG:** * **Anti-Scl-70 (Anti-topoisomerase I):** Specific for Diffuse Systemic Sclerosis; associated with pulmonary fibrosis [1]. * **Anti-Centromere:** Specific for **Limited** Systemic Sclerosis (**CREST syndrome**); associated with digital ischemia and pulmonary hypertension [1]. * **Anti-RNA Polymerase III:** Associated with diffuse skin involvement and increased risk of **Scleroderma Renal Crisis**. * **Nucleolar ANA Pattern:** Always think of Systemic Sclerosis (Anti-fibrillarin, Anti-Th/To, or Anti-PM-Scl). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 236-239.

Question 1

A 19-year-old man with a history of recent-onset asthma presents with chest pain, intermittent claudication, and respiratory distress that is unresponsive to bronchodilators and antibiotics. Physical examination reveals mild hypertension (blood pressure = 150/100 mm Hg), bilateral wheezing, and numerous purpuric skin lesions on the feet. Laboratory studies demonstrate that leukocytes are increased to 14,000/mL with increased eosinophils and platelets are increased to 450,000/mL. BUN is elevated to 30 mg/dL, and serum creatinine is elevated to 3.5 mg/dL. The serum antineutrophil cytoplasmic antibody test is positive. Urinalysis discloses 3+ proteinuria and RBCs. A renal biopsy demonstrates vasculitis of medium-sized arteries, accompanied by eosinophilia. Which of the following is the most likely diagnosis?

Accepted Answer

Churg-Strauss disease

Answer

Henoch-Schonlein purpura

Answer

Loeffler syndrome

Answer

Wegener granulomatosis

Question 2

All may be associated with Sicca syndrome except?

Accepted Answer

Midline granuloma

Answer

Scleroderma

Answer

Rheumatoid arthritis

Answer

Lymphadenopathy

Question 3

Which of the following is NOT a feature of seronegative spondyloarthropathy?

Accepted Answer

Symmetrical Polyarthritis

Answer

Strong association with HLA B27

Answer

Negative Rheumatoid Factor

Answer

Enthesitis

Question 4

A barium swallow is performed in a 44-year-old female who has had difficulty swallowing for months. Radiographically, there is marked dilation of the esophagus with beaking in the distal portion where marked luminal narrowing exists. A biopsy of the lower esophagus shows prominent submucosal fibrosis without much inflammation. What is the most likely cause?

Accepted Answer

CREST syndrome

Answer

Portal hypertension

Answer

Iron deficiency

Answer

Barrett esophagus

Question 5

Which of the following statements about fibromyalgia is true?

Accepted Answer

SPECT studies have shown reduced blood flow to the thalamus

Answer

More common in males

Answer

Sleep EEG studies have shown disruption in REM sleep

Answer

Increased cortisol response to stress

Question 6

A 14-year-old girl on exposure to cold has pallor of extremities followed by pain and cyanosis. In later life, she is prone to develop which of the following conditions?

Accepted Answer

Scleroderma

Answer

Systemic lupus erythematosus

Answer

Rheumatoid arthritis

Answer

Histiocytosis

Question 7

A 24-year-old male presents with abdominal pain, rashes, palpable purpura, and arthritis. What is the most probable diagnosis?

Accepted Answer

Henoch-Schönlein Purpura (HSP)

Answer

Sweet syndrome

Answer

Meningococcemia

Answer

Hemochromatosis/Kawasaki Disease

Question 8

Churg-Strauss syndrome involves which of the following, except?

Accepted Answer

Heart

Answer

Lungs

Answer

Kidneys

Answer

Small blood vessels

Question 9

Mononeuritis multiplex is a major presenting feature of which of the following conditions?

Accepted Answer

Systemic necrotising vasculitis

Answer

Wagner's granulomatosis

Answer

Hypersensitivity vasculitis

Answer

Mucocutaneous lymphnode syndrome

Question 10

Anti-fibrillarin antibodies are associated with which condition?

Accepted Answer

Systemic Sclerosis

Answer

Rheumatoid Arthritis

Answer

Systemic Lupus Erythematosus

Answer

Mixed Connective Tissue Disease

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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